Carcinoid tumour research

All treatments must be fully researched before they can be adopted as standard treatment for everyone. This is so that

• We can be sure they work
• We can be sure they work better than the treatments available at the moment
• We know they are safe

First of all, treatments are developed and tested in laboratories. For ethical and safety reasons, experimental treatments must be tested in the laboratory before they can be tried in patients. If a treatment described here is said to be at the laboratory stage of research, it is not ready for patients and is not available either as NHS treatment or in private health care.

Tests in patients are called clinical trials. In the trials and research section of CancerHelp UK you can find out about the 4 phases of clinical trials. You can also visit our searchable database of clinical trials to find trials for carcinoid tumour. Type 'carcinoid' into the free text search box. If you are interested in a particular trial, print off that page and take it to your own specialist. If the trial is suitable for you, your doctor will need to make the referral to the research team. The database also has information about closed trials and trial results. On the database you can also find out about trials that are closed and about trial results.

There tend to be fewer trials for rare cancers like carcinoid. This is because it is a relatively rare illness so there are fewer people to take part in the trials.

The new treatments we cover here are still being researched. Until studies are completed and we know that they definitely work, these potential new treatments can’t be used as standard therapy for carcinoid.

Researchers are trying to find out more about the genetic causes of carcinoid tumours in the digestive system, including multiple endocrine neoplasia, type I (MEN1) and neurofibromatosis. They hope this will help them to develop ways of preventing carcinoid.

MEN1 is a rare inherited condition. Affected family members have a very high risk of getting tumours of the pituitary and parathyroid glands, the pancreas and, more rarely, carcinoid tumours. Some studies estimate that inherited faulty MEN1 genes are responsible for about 1 in 10 carcinoid tumors (10%). Most of these are stomach carcinoids.

Children have a 50/50 chance of inheriting MEN1 from an affected parent. If your family has the MEN1 syndrome, you can talk to your doctor about the pros and cons of testing for it. We know which gene is responsible for MEN1 syndrome, but genetic testing for MEN1 is only available in a few places. The test is not always 100% accurate and so it is important that you also have genetic counselling to help you make sense of the results.

Neurofibromatosis is a condition that usually runs in families. Affected family members develop many non cancerous tumours, called neurofibromas. These form in nerves under the skin and in other parts of the body. Some people with this condition also develop carcinoid tumours of the small bowel.

The outlook and treatment for carcinoid in the lung is much better than for the more common types of lung cancer (carcinomas). So accurate diagnosis is important. To help tell the difference, researchers are developing tests that can pick up specific substances found in carcinoid cells, but not in lung cancer cells. Other substances may be found in both carcinoids and carcinomas, but higher levels are found in one type. Most of these tests involve treating tissue samples with special antibodies produced in the laboratory. The antibodies are designed to recognise specific substances in certain types of tumours.

There is a study looking at a possible new way of finding and monitoring neuroendocrine tumour (NETs), including carcinoid tumours. Some NETs can produce hormones or chemicals called markers. Tumour markers can help to show whether the tumour is growing and whether treatment is working. This study is looking at whether cancer cells that break away from the original tumour and circulate in the bloodstream could be a possible new marker for NETs.

We know that surgery can cure early stage carcinoid. Treatment is more difficult for carcinoids that have spread to another part of the body. A combination of chemotherapy drugs can help to control the cancer and any symptoms it causes. The combination doctors usually use is

• Streptozocin
• Cisplatin
• 5 fluorouracil

The NET 01 trial in the UK is looking at a new combination of treatment for people with carcinoid that has spread or can't be removed. This replaces the 5 fluorouracil with a drug called capecitabine (Xeloda). This phase 2 trial aims to find out which treatment works best and what the side effects of the treatment are. It has now closed and we are waiting for the results. The results will be used to design a phase 3 trial. 

Another phase 2 trial (the NET trial) is looking at giving the drug capecitabine (Xeloda) on its own for people who have carcinoid that has spread and can't be removed. The trial has closed and we are waiting for the results.

You can find out about these chemotherapy trials for carcinoid on our clinical trials database. To find them, type 'carcinoid' into the free text search box. Or pick ‘neuroendocrine’ from the dropdown menu of cancer types.

New chemotherapy drugs are being tested to find treatments that are active against carcinoids. One such drug is called 7-hydroxytryptophan (high-drox-ee-trip-toe-fan). This chemical killed carcinoid tumour cells in test tube experiments in the lab. But it will be some time before this drug is studied in patients.

In America, researchers are trying to find out whether adding biological therapy to chemotherapy makes the chemotherapy work better for carcinoid tumours of the digestive system. A drug called bevacizumab (Avastin) is being added to the combination chemotherapy FOLFOX. FOLFOX uses the chemotherapy drugs fluorouracil, leucovorin (calcium folinate), and oxaliplatin. Bevacizumab is a monoclonal antibody that may stop cancer growth by blocking blood flow to the tumour. Carcinoid tumours seem to depend on blood vessels to grow. So doctors think a treatment like bevacizumab may stop carcinoids growing. At the moment it is only being used in America to treat carcinoid tumours that have spread (metastatic carcinoid).

Also in America, there has been some research into a new biological therapy drug called atiprimod. This has been investigating treatment for patients with slow growing carcinoid of the digestive system. Atiprimod is a type of drug that blocks blood vessel growth. These drugs are called anti angiogenics because angiogenesis means ‘growth of blood vessels’. Patients in the study have carcinoid that has spread and either

• Have symptoms such as diarrhoea, flushing, and wheezing not controlled by standard therapy (octreotide)
• Have carcinoid that is growing

Everolimus is a type of cancer growth inhibitor which stops signals in the cells that make them grow and divide. A phase 3 trial has been comparing giving everolimus with octreotide to octreotide with a placebo for people with advanced carcinoid. The researchers found that the everolimus with octreotide controlled the cancer growth for longer, just over 16 months compared to just over 11 months.

The RADIANT 4 trial is looking at everolimus for non functioning neuroendocrine tumours of the digestive system or lungs.

Somatostatin analogues aim to reduce the symptoms produced by some carcinoid tumours. Octreotide is the most commonly used somatostatin analogue drug. 

Pasireotide is a new type of somatostatin analogue. An American and European phase 2 clinical trial looked at pasireotide for carcinoid of the digestive system. It showed that pasireotide can control the symptoms of diarrhoea and flushing in more than 1 in 4 people (27%) with carcinoid rumours that had spread. These people had already tried other types of somatostatin analogue and they no longer worked. Pasireotide improved their quality of life. The SIG-NET trial is comparing pasireotide with high dose octreotide for people who have carcinoid syndrome symptoms that are not controlled with other drugs. 

There is a trial looking at lanreotide injections for neuroendocrine tumours of the digestive system. You may be able to join this trial if you have a carcinoid tumour in the stomach, large or small bowel, or pancreas, and it cannot be removed surgically. You have 24 injections over 2 years. These will either be lanreotide or dummy (placebo) injections. Doctors want to find out more about how well lanreotide can slow down or stop the growth of the tumour, and what the side effects are. 

You can find out more about these trials on our clinical trials database.

Carcinoid is a slow growing tumour and its symptoms can affect your quality of life. But treatments can also affect your quality of life. As carcinoid is often a slow growing chronic condition, doctors don’t want to give people treatments that make them feel worse instead of better. In the carcinoid quality of life study, researchers are interviewing a large number of people with carcinoid, who are having a variety of different treatments. They hope to find out how the different treatments affect peoples’ quality of life. You can find out more about this trial on our clinical trials database by typing NET into the search box and ticking the box for trials that have finished recruiting.